Top Docs 2012: Matters of the Heart

Plumbing Problems

Hypoplastic left heart syndrome is a deadly congenital heart defect—and it’s more common than you might think.

Dr. James Jaggers likes a challenge. And that’s a good thing, considering the 12 years of postgraduate training he had to complete to do the extremely complicated job he does at Children’s Hospital Colorado. Jaggers is a pediatric cardiothoracic surgeon, which means he takes the hearts of infants into his hands and fixes them every day.

If that doesn’t give you a God complex, nothing will. But Jaggers is humble when he describes how he repairs a life-threatening condition called hypoplastic left heart syndrome (HLHS). “You gotta just do some plumbing really,” he says. “It’s rerouting flows and enlarging tubes. It’s not the most difficult surgery we do, but these HLHS kids are just so sick.”

Babies with HLHS are born with an underdeveloped left side of the heart, which prevents the muscle from pumping enough blood to the body. Without surgery, 90 percent die within one month; 100 percent die within one year. For many years, cardiac-care doctors thought the best way to treat HLHS was an immediate heart transplant. Unfortunately, the average time to receive a pediatric heart transplant is between three and six months. The math just didn’t add up. Instead, surgeons began trying to fix the abnormality surgically, and ultimately figured out that a series of three procedures—in order, the Norwood, the Glenn, and the Fontan procedures—could remedy the problem.

Although the first Norwood procedure was executed in the early 1980s, the procedure was still incurring mortality rates in the 43 percent range well into the late 1990s. Today, many respectable surgical departments are routinely seeing 85 percent survival rates. At Children’s Hospital Colorado, Jaggers and company are hovering around 93 percent, one of the best rates in the country.

A Patient's Story

Every expectant mother has a love-hate relationship with the 20-week ultrasound appointment. If everything goes well, a mother can find out just how big her baby is getting, the position of the fetus, and whether it’s a boy or a girl. But if everything doesn’t go well, the fun stuff can fade into the background when the technician says something just doesn’t look quite right.

The latter scenario sounds familiar to Teresa Beck, a 28-year-old Loma, Colorado, resident. Pregnant with her first child, Beck was sent to see a specialist when her ultrasound tech told her she wasn’t able to see everything like she wanted to. After visiting with a high-risk pregnancy doctor, Beck had a confirmed diagnosis for her growing baby: hypoplastic left heart syndrome.

Because the HLHS diagnosis was made in utero—about 80 percent are—Beck had to decide whether she wanted to keep the baby. She knew she needed more information. Beck went to Children’s Hospital Colorado. It was there, on December 5, 2011, that she met the crowd of health-care professionals—including Dr. James Jaggers—who could save her child’s life. She decided to have the baby. “There was just no way I could live with the questions,” Beck says. “What if the doctors were wrong? What if it wasn’t as bad as they thought? I decided there must be a reason I was pregnant with a heart baby.”

Because babies with HLHS often need immediate medical intervention after birth, Jaggers wanted Beck to deliver in Denver. So she moved to the Mile High City on January 12, 2012. Curtis Steele was born at 2:05 a.m. on January 23 at University of Colorado Hospital. By 6 a.m. on January 24, one-day-old Curtis was at Children’s Hospital Colorado for emergency surgery to repair complications with some veins surrounding his tiny heart. Eight days later, Jaggers took Curtis to the operating room for the Norwood procedure.

“Curtis came out of the womb looking pretty good,” Jaggers says. “He continued to do really well through the vein surgery and the Norwood.” He did so well, Beck says, the cardiac intensive care unit nurses took to calling Curtis “the rock star baby.”

Of course, even rock stars have ups and downs. Curtis had a few complications, but he was eventually discharged from Children’s in early March 2012. Beck took him home to Loma.

“It’s been nonstop since then,” Beck says. “It’s a full-time job to care for an infant with a heart condition.” She isn’t complaining. Although she will not know the luxury of having a “normal” baby this time around, she says Curtis is doing well and is, of course, a momma’s boy.

Beck took Curtis back to Jaggers for the Glenn procedure in early summer 2012. The Fontan surgery is years away. Curtis’ short-term prognosis is good, though kids with HLHS who undergo this series of procedures do have a much higher risk of eventually developing heart failure as teenagers or adults. But for now, that is a distant worry for Beck. “I don’t know what I would’ve done without Dr. Jaggers,” Beck says. “He’s a miracle worker, and he knows how to make us feel secure in our decisions and our ability to handle all of this.”